| Note | epidermodysplasia verruciformis (EV) is a model of malignant transformation from benign cutaneous viral lesion. |
| Phenotype and clinics | age at onset is variable; more frequently: young adults or children two types of elementary cutaneous lesions are observed :
- persistant papule-like warts, isolated, or confluent with a psoriasic aspect
- white spots, pityriasis versicolor-like
both types of lesions are localized mainly on the outer part of the hands, on foreharms, legs, face, trunk and perianal zone. immunodeficiency: decreased immune response of T lymphocytes to mitogens, decreased humoral response to Human Papilloma Virus antigens; EV lesions have been described in renal transplant recipients. various Human Papilloma Virus (HPV) subtypes are regularly detected in the cutaneous lesions: HPV 5, 8, 9, 12, 14, 15, 17, 19, 25, 36, 38, 47, 50. patients are simultaneously affected by differents HPV subtypes, according to disease localisation; these subtypes are different from those observed in common warts (HPV 2, 3, 4, 10). |
| Neoplastic risk | risk of malignant transformation of cutaneous lesions is within a delay of 20 to 30 yrs (very slow process comparable to the genital carcinogenesis associated with high risk HPVs) cytology: squamous cell carcinoma (spinocellular or basocellular carcinoma, Bowen disease). correlation with oncogenic subtypes of HPV found in the transformed lesions: HPV 5, 8, 14; the most frequent subtypes are HPV 5 and 8 (90% of cases). benign familial forms are associated with HPV 3, without malignant evolution. HPV-5 is present in the macular lesions. probable potentialisation by UV ligth: 25 to 30% of malignant lesions localised to the face and forehead (hypothetic role of P53 mutations). protein E6 and/or E7 (tumor suppressor function) from HPV seem to be involved in the malignant transformation. |
| Treatment | surgical resection of localized lesions; chemotherapy with Acitretin (25 mg/j) for multifocal lesions. |
| Evolution | local recidives, enhanced by UV exposition. |
| Human papillomaviruses are commonly found in normal skin of immunocompetent host. |
| Astori G, Lavergne D, Benton C, Hockmayr B. Egawa K, Garbe C, de Villiers EM. |
| J invest Dermatol 1998; 110: 752-5. |
| PMID 98239143 |
| |
| Detection of new human papillomavirus sequences in skin lesions of a renal transplant recipient and characterization of one complete genome related to epidermodysplasia verruciformis-associated types. |
| Bens G, Wieland U, Hofmann A, Hopfl R, Pfister H. |
| J Gen Virol 1998; 79: 779-87. |
| PMID 98227928 |
| |
| Epidermodysplasia verruciformis with numerous squamous cell carcinomas. |
| Bonvalet D, Blanchet-Bardon C, Verola O. |
| Nouv Dermatol 1997; suppl1: S1-20. |
| |
| Psoriasis: A possible reservoir for human papillomavirus type 5, the virus associated with skin carcinomas of epidermodysplasia verruciformis. |
| Favre M, Orth G, Majewski S, Balool S, Pura A, Jablonska S. |
| J Invest Dermatol 1998; 110: 311-7. |
| PMID 98200036 |
| |
| E7 proteins of four groups of papillomaviruses, irrespective of their tissue tropism or cancer association, possess the ability to transactivate transcriptional promoters E2F site dependently. |
| Hiraiwa A, Kiyono T, Suzuki S, Ohashi M, Ishibashi M. |
| Virus Genes 1996; 12: 27-35. |
| PMID 97033371 |
| |
| Human papillomavirus DNA in non-melanoma skin cancers of a renal transplant recipient: detection of a new sequence related to epidermodysplasia verruciformis associated types. |
| Hopfl R, Bens G, Wieland U, Petter A, Zelger B, Fritsch P, Pfister H. |
| J Invest Dermatol 1997; 108: 53-6. |
| PMID 97134727 |
| |
| p53 mutations implicate sunlight in post-transplant skin cancer irrespective of human papillomaviruss status. |
| McGregor JM, Berkhout RJ, Rozycka M, ter Schegget J, Bouwes Bavinck JN, Brooks L, Crook T. |
| Oncogene 1997; 15: 1737-40. |
| PMID 98007664 |
| |
| Epidermodysplasia verruciformis as a model of human papillomavirus-induced genetic cancer of the skin. |
| Majewski S, Jablonska S. |
| Arch of Dermatol 1995; 131: 1212-8. |
| PMID 96067820 |
| |
| Human papillomavirus-associated tumors of the skin and mucosa. |
| Majewski S, Jablonska S. |
| J Am Acad Dermatol 1997; 36: 659-85. |
| PMID 97291988 |
| |
| Skin autografts in epidermodysplasia verruciformis : human papillomavirus-associated cutaneous changes need over 20 years for malignant conversion. |
| Majewski S, Jablonska S. |
| Cancer Res 1997; 57: 4214-6. |
| PMID 97470618 |
| |
| Regulatory interactions of transcription factor YY1 with control sequences of the E6 promoter of human papillomavirus type 8. |
| Pajunk HS, May C, Pfister H, Fuchs PG. |
| J Gene Virol 1997; 78: 3287-95. |
| PMID 98062151 |
| |
| HPV-5 typing with nested PCR and sequencing in Epidermodysplasia verruciformis. |
| Schaller J, Rohwedder A, Fuchs M, Maron A, Kunze J. |
| Hautarzt 1996; 47: 454-8. |
| PMID 96293936 |
| |
| E2 represses the late gene promoter of human papillomavirus type 8 at high concentrations by interfering with cellular factors. |
| Stubenrauch F, Leigh IM, Pfister H. |
| J Virol 1996; 70: 119-26. |
| PMID 96099421 |
| |